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LETTER TO EDITOR
Year : 2015  |  Volume : 63  |  Issue : 5  |  Page : 794-796

C1-2 ganglioneuroma in a patient with neurofibromatosis type-1


Department of Neurosurgery, Sir J.J. Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India

Date of Web Publication6-Oct-2015

Correspondence Address:
Hrushikesh Kharosekar
Department of Neurosurgery, Sir J.J. Group of Hospitals and Grant Medical College, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.166550

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How to cite this article:
Kharosekar H, Valsangkar S, Velho V. C1-2 ganglioneuroma in a patient with neurofibromatosis type-1. Neurol India 2015;63:794-6

How to cite this URL:
Kharosekar H, Valsangkar S, Velho V. C1-2 ganglioneuroma in a patient with neurofibromatosis type-1. Neurol India [serial online] 2015 [cited 2019 Oct 21];63:794-6. Available from: http://www.neurologyindia.com/text.asp?2015/63/5/794/166550


Sir,

Ganglioneuromas (GNs) are rare benign neurogenic tumors arising from neural crest cells. The most commonly affected sites are the posterior mediastinum, the retroperitoneum, and the adrenal gland. GNs often present as a solitary, painless, and slow-growing mass, and occurrences in the cervical region are extremely rare. Only 11 cases of cervical GNs have been reported till date in literature, with only 5 cases associated with neurofibromatosis type-1 (NF-1). We report the 6th case of a cervical GN in a patient with NF-1.[1]

A 32-year-old male patient was referred to us with complaints of gradually progressive weakness in all four limbs for the past 8 months. Physical examination demonstrated several cafe au lait spots on his trunk. There was no family history of NF. Neurological examination revealed weakness of all four limbs (4/5). Deep tendon reflexes were hyperactive with positive Babinski sign. Magnetic resonance imaging (MRI) of the cervical spine showed right sided extramedullary dumbbell-shaped mass at the C1 level extending outside through the neural foramen. The mass compressing spinal cord was isointense on T1-weighted image (T1WI) and showed slightly increased signal intensity on gadolinium-enhanced T1WI. T1W and T2W sagittal images revealed the severely compressed spinal cord [Figure 1] and [Figure 2]. Computed tomography (CT) brain of this patient revealed left sphenoidal dysplasia [Figure 3]. The patient underwent C1 laminectomy with complete excision of the lesion, with decompression of the spinal cord, in a prone position through a posterior midline incision. The mass, which was significantly indenting the dura, was solid, soft, non-vascular, and extramedullary in location. Histopathology revealed an encapsulated tumor arranged in compact interlacing fascicles and bundles. Spindle-shaped cells with scant cytoplasm had oval to elongated nuclei. The tumor also showed numerous ganglion cells with an eosinophilic cytoplasm suggestive of a GN. The postoperative course was uneventful with improvement in the patient's power to 5/5 at discharge [Figure 4] and [Figure 5].
Figure 1: (a and b) Magnetic resonance imaging cervical spine with contrast. T1-weighted images showing an isointense lesion and T2-weighted images showing an iso-hyperintense lesion compressing the cord

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Figure 2: Axial cuts of magnetic resonance imaging cervical spine showing both the extradural and intradural extramedullary components

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Figure 3: Computed tomography angiogram showing sphenoid dyplasia

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Figure 4: (a and b) Postoperative magnetic resonance imaging cervical spine at 3-month follow-up showing complete resection and no recurrence

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Figure 5: Histopathological slide showing an encapsulated tumor arranged in compact interlacing fascicles and bundles. Spindle-shaped cells with scant cytoplasm had oval to elongated nuclei. The tumor also showed numerous ganglion cells with eosinophilic cytoplasm

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Nerve sheath tumors can be intradural (70–75%), extradural (15%), or extradural–intradural dumbbell masses (15%). The distribution of nerve sheath tumors throughout the spine is uniform with a slight lumbar predominance; and, multiple lesions are reported in 1–55% of patients and are common in patients with NF.[2] These tumors are isointense compared to the spinal cord on T1WI, and most of them are iso-to-hyperintense on T2WI. The nonhomogeneous appearance and a low-to-intermediate intensity on T1W1 image and a high signal intensity on T2W1 images corresponds to areas of cystic degeneration, hemorrhage, or necrotic degeneration. All nerve sheath tumors show enhancement following contrast administration. GNs histologically show mature ganglion cells, Schwann cells, and nerve fibers and have a distinct whorled pattern. The extradural nerve sheath tumors have to be differentiated from a vertebral chordoma, both of which are typically slow-growing and are extending through and expanding the neural foramina. Some of the GNs (25%) are not truly benign but contain poorly differentiated components including ganglioneuroblastomas, neuroblastomas, or pheochromocytomas. If a GN has atypical CT or MR features, then coexistence of a malignant component should be considered along with the ganglionic components.

GN is most commonly located in the retroperitoneum and posterior mediastinum. A solitary GN at the cervicothoracic region arising from the cervical nerve root within the intervertebral foramen is extremely uncommon. Among the 11 previously published cases of cervical GN that were producing spinal cord compression, only five were associated with NF-1.[1] An association with NF-1 has been speculated in cases with multiple occurrences but genetic associations between NF-1 and GN remain unclear. GNs are usually encapsulated masses with a firm consistency; complete surgical resection is the best option for a solitary GN. Prognosis is generally favorable considering that GNs, the most differentiated form of neuroblastic tumor, have no metastatic potential. However, local recurrence, regional lymph node metastasis, and malignant transformation into malignant peripheral nerve sheath tumors have been reported; therefore, periodic radiologic surveillance should be performed after resection.[1],[3]

We described a rare case of GN of the cervical spine in a patient with NF-1. We performed total resection of the dumbbell tumor which is the best option for a solitary GN.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Son DW, Song GS, Kim YH, Lee SW. Ventrally located cervical dumbbell ganglioneuroma producing spinal cord compression. Korean J Spine 2013;10:246-8.  Back to cited text no. 1
    
2.
Moorthy N, Bhaskar G, Venkatakumar N, Sharada V. Cervical ganglioneuroma. Indian J Radiol Imaging 2001;11:220-1.  Back to cited text no. 2
  Medknow Journal  
3.
Hioki A, Miyamoto K, Hirose Y, Kito Y, Fushimi K, Shimizu K. Cervical symmetric dumbbell ganglioneuromas causing severe paresis. Asian Spine J 2014;8:74-8.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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